In 2005, the first case of variant Creutzfeldt-Jakob disease (vCJD) wasreported in Spain, in a woman born in 1978 with clinical onset of symptomsin 2004 [1]. She subsequently died in 2005.

Recently, two more laboratory-confirmed vCJD cases were reported to theSpanish CJD state registry. In February 2006, a woman born in 1957 developedprogressive cognitive deterioration, and died in December 2007 withsuspected sporadic CJD (typical EEG in October 2007) MM at codon 129 and nomutations in PRPN gene. A man born in 1967 had onset in May 2007 withpsychiatric symptoms, and after several months developed progressivecognitive decline with dementia, typical MRI, MM at codon 129, no mutationsin PRPN gene. He died in February 2008. Post-mortem, neuropathology withhistochemistry confirmed vCJD in both cases. No clear specific dietaryhabits, blood donations or reception were recorded. Neither case appears tohave visited the United Kingdom before 2004.

The latest two cases were resident in the same region of the country,Castilla y Leon, but no link between them was established.